Hesford James, Medford Andrew Rl, Gunawardena Harsha
North Bristol NHS Trust, Bristol, UK.
North Bristol Lung Centre, Bristol, UK;
Clin Med (Lond). 2021 Mar;21(2):e231-e233. doi: 10.7861/clinmed.2020-1037.
We describe the case of a 61-year-old woman who presented with progressive respiratory symptoms and imaging demonstrating multiple opacities in the right lung with hilar and mediastinal lymphadenopathy suggestive of multifocal adenocarcinoma. Subsequent biopsies were consistent with focal changes of organising pneumonia (OP) and no evidence of malignancy. She was treated with steroids for cryptogenic OP with limited response. There was clinical and radiological progression with new lung nodules, mediastinal and thoracic spinal canal infiltration. There was ongoing concern that clinical findings represented disseminated malignancy. Following further investigation and multidisciplinary respiratory and rheumatology review, a diagnosis of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) - granulomatosis with polyangiitis (GPA) was confirmed. The case highlighted the multisystem nature of GPA with unusual dural and large vessel aortic and pulmonary trunk involvement.
我们描述了一名61岁女性的病例，她出现进行性呼吸症状，影像学显示右肺有多个混浊影，伴有肺门和纵隔淋巴结肿大，提示多灶性腺癌。随后的活检结果与机化性肺炎（OP）的局灶性改变一致，未发现恶性肿瘤证据。她接受了类固醇治疗隐源性OP，但反应有限。出现了新的肺结节、纵隔和胸段椎管浸润，临床和影像学均有进展。人们一直担心临床发现代表播散性恶性肿瘤。经过进一步检查以及呼吸科和风湿科的多学科会诊，确诊为抗中性粒细胞胞浆抗体（ANCA）相关血管炎（AAV）——肉芽肿性多血管炎（GPA）。该病例突出了GPA的多系统性质，伴有不寻常的硬脊膜以及大血管主动脉和肺动脉主干受累。
